Long-term outcomes in total colonic aganglionosis: A 32-year experience

Mauricio A. Escobar, Jay L. Grosfeld, Karen W. West, L. R. Scherer, Thomas M. Rouse, Scott A. Engum, Frederick J. Rescorla

Research output: Contribution to journalArticle

60 Citations (Scopus)

Abstract

Background/Methods: A 32-year retrospective review from 1972 to 2004 analyzed complications and long-term outcomes in children with total colonic aganglionosis (TCA) as they relate to the procedure performed. Results: Thirty-six patients (27 boys, 9 girls) had TCA. The level of aganglionosis was distal ileum (26), mid-small bowel (8), midjejunum (1), and entire bowel (1). Enterostomy was performed in 35 of 36. Eight developed short bowel syndrome. Twenty-nine (81%) had a pull-through at 15 ± 6 months (modified Duhamel 20, Martin long Duhamel 4, and Soave 5). Six had a Kimura patch. Postoperative complications (including enterocolitis) were more common after long Duhamel and Soave procedures. Seven (19%; 2 with Down's syndrome) died (3 early, 4 late) from pulmonary emboli (1), sepsis (1), fluid overload (1), viral illness (1), liver failure (1), arrhythmia (1), and total bowel aganglionosis (1). Mean follow-up was 11 ± 9 years (range, 6 months-29 years). Twenty-four (83%) of 29 patients exhibited growth by weight of 25% or more, 21 (91%) of 23 older than toddler age had 4 to 6 bowel movements per day, and 17 (81%) of 21 were continent. In 5 of 6, the Kimura patch provided functional benefit with proximal disease. Conclusion: Long-term survival was 81%. The highest morbidity occurred with long Duhamel or Soave procedures. The modified Duhamel is our procedure of choice in TCA. Bowel transplantation is an option for TCA with unadapted short bowel syndrome.

Original languageEnglish (US)
Pages (from-to)955-961
Number of pages7
JournalJournal of Pediatric Surgery
Volume40
Issue number6
DOIs
StatePublished - Jun 2005

Fingerprint

Hirschsprung Disease
Short Bowel Syndrome
Enterostomy
Enterocolitis
Liver Failure
Down Syndrome
Embolism
Ileum
Cardiac Arrhythmias
Sepsis
Transplantation
Morbidity
Weights and Measures
Lung
Survival
Growth

Keywords

  • Hirschsprung's disease
  • Kimura patch
  • Modified Duhamel pull-through
  • Total colonic aganglionosis

ASJC Scopus subject areas

  • Surgery

Cite this

Escobar, M. A., Grosfeld, J. L., West, K. W., Scherer, L. R., Rouse, T. M., Engum, S. A., & Rescorla, F. J. (2005). Long-term outcomes in total colonic aganglionosis: A 32-year experience. Journal of Pediatric Surgery, 40(6), 955-961. https://doi.org/10.1016/j.jpedsurg.2005.03.043

Long-term outcomes in total colonic aganglionosis : A 32-year experience. / Escobar, Mauricio A.; Grosfeld, Jay L.; West, Karen W.; Scherer, L. R.; Rouse, Thomas M.; Engum, Scott A.; Rescorla, Frederick J.

In: Journal of Pediatric Surgery, Vol. 40, No. 6, 06.2005, p. 955-961.

Research output: Contribution to journalArticle

Escobar, MA, Grosfeld, JL, West, KW, Scherer, LR, Rouse, TM, Engum, SA & Rescorla, FJ 2005, 'Long-term outcomes in total colonic aganglionosis: A 32-year experience', Journal of Pediatric Surgery, vol. 40, no. 6, pp. 955-961. https://doi.org/10.1016/j.jpedsurg.2005.03.043
Escobar MA, Grosfeld JL, West KW, Scherer LR, Rouse TM, Engum SA et al. Long-term outcomes in total colonic aganglionosis: A 32-year experience. Journal of Pediatric Surgery. 2005 Jun;40(6):955-961. https://doi.org/10.1016/j.jpedsurg.2005.03.043
Escobar, Mauricio A. ; Grosfeld, Jay L. ; West, Karen W. ; Scherer, L. R. ; Rouse, Thomas M. ; Engum, Scott A. ; Rescorla, Frederick J. / Long-term outcomes in total colonic aganglionosis : A 32-year experience. In: Journal of Pediatric Surgery. 2005 ; Vol. 40, No. 6. pp. 955-961.
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abstract = "Background/Methods: A 32-year retrospective review from 1972 to 2004 analyzed complications and long-term outcomes in children with total colonic aganglionosis (TCA) as they relate to the procedure performed. Results: Thirty-six patients (27 boys, 9 girls) had TCA. The level of aganglionosis was distal ileum (26), mid-small bowel (8), midjejunum (1), and entire bowel (1). Enterostomy was performed in 35 of 36. Eight developed short bowel syndrome. Twenty-nine (81{\%}) had a pull-through at 15 ± 6 months (modified Duhamel 20, Martin long Duhamel 4, and Soave 5). Six had a Kimura patch. Postoperative complications (including enterocolitis) were more common after long Duhamel and Soave procedures. Seven (19{\%}; 2 with Down's syndrome) died (3 early, 4 late) from pulmonary emboli (1), sepsis (1), fluid overload (1), viral illness (1), liver failure (1), arrhythmia (1), and total bowel aganglionosis (1). Mean follow-up was 11 ± 9 years (range, 6 months-29 years). Twenty-four (83{\%}) of 29 patients exhibited growth by weight of 25{\%} or more, 21 (91{\%}) of 23 older than toddler age had 4 to 6 bowel movements per day, and 17 (81{\%}) of 21 were continent. In 5 of 6, the Kimura patch provided functional benefit with proximal disease. Conclusion: Long-term survival was 81{\%}. The highest morbidity occurred with long Duhamel or Soave procedures. The modified Duhamel is our procedure of choice in TCA. Bowel transplantation is an option for TCA with unadapted short bowel syndrome.",
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AU - Rescorla, Frederick J.

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AB - Background/Methods: A 32-year retrospective review from 1972 to 2004 analyzed complications and long-term outcomes in children with total colonic aganglionosis (TCA) as they relate to the procedure performed. Results: Thirty-six patients (27 boys, 9 girls) had TCA. The level of aganglionosis was distal ileum (26), mid-small bowel (8), midjejunum (1), and entire bowel (1). Enterostomy was performed in 35 of 36. Eight developed short bowel syndrome. Twenty-nine (81%) had a pull-through at 15 ± 6 months (modified Duhamel 20, Martin long Duhamel 4, and Soave 5). Six had a Kimura patch. Postoperative complications (including enterocolitis) were more common after long Duhamel and Soave procedures. Seven (19%; 2 with Down's syndrome) died (3 early, 4 late) from pulmonary emboli (1), sepsis (1), fluid overload (1), viral illness (1), liver failure (1), arrhythmia (1), and total bowel aganglionosis (1). Mean follow-up was 11 ± 9 years (range, 6 months-29 years). Twenty-four (83%) of 29 patients exhibited growth by weight of 25% or more, 21 (91%) of 23 older than toddler age had 4 to 6 bowel movements per day, and 17 (81%) of 21 were continent. In 5 of 6, the Kimura patch provided functional benefit with proximal disease. Conclusion: Long-term survival was 81%. The highest morbidity occurred with long Duhamel or Soave procedures. The modified Duhamel is our procedure of choice in TCA. Bowel transplantation is an option for TCA with unadapted short bowel syndrome.

KW - Hirschsprung's disease

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KW - Total colonic aganglionosis

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